Critical Airway Stenosis in an Adolescent Male With Pompe Disease and Thoracic Lordosis: A Case Report.

Autor: Brenn BR; From the Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware., Theroux MT, Shah SA, Mackenzie WG, Heinle R, Scavina MT
Jazyk: angličtina
Zdroj: A & A case reports [A A Case Rep] 2017 Oct 01; Vol. 9 (7), pp. 199-203.
DOI: 10.1213/XAA.0000000000000564
Abstrakt: An adolescent male with late-onset Pompe disease (glycogen storage disease type II) presented with a history of restrictive airway disease and a near-cardiorespiratory arrest during anesthesia for a liver biopsy initially thought to be due to bronchospasm. During a subsequent posterior spinal fusion procedure, he suffered cardiorespiratory arrest resulting in the procedure being aborted. Bronchoscopy performed shortly after resuscitation revealed an undiagnosed narrowing of the distal trachea and bronchi. This is the first description of a patient with late-onset Pompe disease with undiagnosed critical tracheal stenosis due to the progression of thoracic lordosis, which was ultimately relieved by posterior spinal fusion.
Databáze: MEDLINE